Langerhans Cell Histiocytosis in Sphenoid Sinus: Uncommon Bone Involvement.

BACKGROUND Langerhans cell histiocytosis (LCH) is a rare and uncontrolled proliferation of dendritic cells of myeloid origin. The incidence of LHC was estimated at 5 cases per million children ages 0-15 years old. The most common places for this tumor are the jaw, vertebra, pelvis, and the extremities. The disease with multisystem involvement can present a mortality rate of 20% and one-third of children have multisystem involvement. We present a case with unusual bone involvement of the anterior cranial base with a challenging diagnosis and a complex surgical approach. CASE REPORT We report the case of a 6-year-old boy who manifested the disease with daily holocranial headache, worse in the frontal region and refractory to analgesia for 10 days, strabismus homonymous, diplopia, and right palpebral ptosis. The tumor affected the sphenoid sinus, internal carotid artery, and sella turcica, and made contact with the pituitary gland. A joint surgery with Otorhinolaryngology and Neurosurgery was performed by nasal endoscopic access to the skull base by means of the right medial turbinectomy (for the access) and right sphenoid opening, septectomy and opening of the left sphenoid to work with 4 hands and, after resection of lesion, inside the sphenoid. CONCLUSIONS This patient had rare bone involvement from LCH and atypical clinical presentation next to the important and delicate structures of the anterior skull base, but had a satisfactory outcome.

Intracranial thrombosis after ventriculoperitoneal shunting.

We report a case of intracranial thrombosis (IT) after ventriculoperitoneal shunting (VPS). We reviewed the literature to highlight the importance of considering the possibility of cerebral venous thrombosis following VPS, even though it is a rarely reported complication. A 14-year-old boy underwent distal catheter replacement due to its short size that was detected during a routine consultation. Five days postoperatively, he experienced nausea, vomiting, seizures, and headache. Although a diagnosis of meningitis was considered, diagnosis of IT was eventually confirmed through computed tomography venography and gadolinium magnetic angioresonance. The patient subsequently underwent anticoagulant therapy, which led to complete resolution of symptoms. In this report, we suggest a possible association between VPS and IT, which is a complication that is not commonly reported. The literature suggests that decreased blood flow due to over-drainage of CSF after VPS causes decreased head pressure, culminating in venous stasis and consequent thrombosis. In addition, the literature describes associations between IT and lumbar puncture (LP); therefore, it is important to consider the possibility of IT following these procedures.

Fetal epidural hematoma secondary to a mild blunt prenatal trauma: case report and review of the literature.

Fetal intracranial hemorrhage affects 1 in every 10,000 pregnancies. In most cases, the etiology of the bleeding is multifactorial, and they can be either related to the mother or the fetus. Blunt prenatal trauma was occasionally associated with these hemorrhages, nevertheless, reports of hematomas secondary to mild traumas are rare. Within the prenatal intracranial bleedings, the most frequent are the subarachnoid hematoma and intraparenchymal, scarcely ever the epidural hematoma. Treating these bleedings is challenging due to the ongoing pregnancy. Thus, the prognosis is often reserved, with a mortality rate of 43% and 25% of neurological sequelae. Here, we report a singular case of a fetal epidural hematoma secondary to a mild blunt trauma at the third trimester with a good outcome.

Hydrocephalus secondary to dengue encephalitis in an infant: case report.

BACKGROUND: Dengue is the leading cause of mosquito-borne viral infection. It is responsible for high morbidity and mortality in children living in endemic areas. Nowadays, neurological complications are progressively referred to and include a broad spectrum of symptoms. It can be secondary to metabolic alterations, direct invasion by the virus, and enhanced autoimmune response. CASE PRESENTATION: Here, we report a rare case of hydrocephalus secondary to dengue encephalitis. A 13-day-old boy was diagnosed with dengue. He evolved with seizures and impaired consciousness being diagnosed with encephalitis. After clinical treatment, he was discharged fully recovered. Three months later, he presented with signs and symptoms of intracranial hypertension. Brain MRI revealed hydrocephalus secondary to acquired aqueduct stenosis. The patient underwent an endoscopic third ventriculocisternostomy. CONCLUSION: Dengue infection is a differential diagnosis for acute febrile neurological impairment in children from endemic areas. Follow-up should be offered after dengue encephalitis to detect possible late-onset complications, as hydrocephalus.

Encefalocele fronto-etmoidal. Abordagem endoscópica / Fronto-ethmoid encephalocele. Endoscopic approach

Introdução: A encefalocele consiste em uma má formação congênita do SNC, caracterizada pela herniação do tecido nervosodecorrente de alterações do tubo neural. Em sua forma intranasal, a encefalocele pode ser confundida com inúmeras afecçõesque mimetizam vários outros sinais e sintomas, contribuindo para diagnósticos errôneos por longos anos. Alguns pacientescursam com quadro clínico agravado, podendo evoluir com quadros de meningites de repetição e abscessos cerebrais, quepodem culminar com prognóstico reservado. Relato de Caso: Paciente de 44 anos de idade, com história de quatro anos depresença de secreção de líquido claro e inodoro unilateral à direita, usualmente diagnosticado como quadros de rinite unilaterale/ou polipose nasal. A TC de seios da face e a RM demostraram encefalocele fronto-etmoidal, sendo submetido a tratamentocirúrgico vídeo-assistido. Discussão: O diagnóstico precoce é a melhor opção a estes pacientes, sendo o tratamento cirúrgicosempre indicado. A escolha do método de tratamento deve buscar aquele que obtenha resultados com menor morbidade possível.Acredita-se que a cirurgia endoscópica é uma excelente opção pelas altas taxas de sucesso e relativa menor morbidade quandocomparada a cirurgia tradicional. Conclusão: A cirurgia endoscópica nasal, com acesso a base do crânio, mostrou-se uma excelente opção terapêutica ao caso reportado.

Lipomas intracranianos / Intracranial lipomas

Os lipomas sao lesoes constituídas por um tecido gorduroso maduro. Apesar de sua grande freqüência no homem, em especial no tecido celular subcutâneo, sao raramente encontrados em localizaçao intracraniana. O corpo caloso é o local mais freqüente, porém já foram descritos casos em quase todos os locais do encéfalo. Freqüentemente sao assintomáticos, porém podem cursar com um quadro de cefaléia, crises convulsivas, alteraçoes mentais ou déficits focais. O tratamento é conservador na maioria dos casos. Os autores apresentam 5 casos de lipomas intracranianos. Em quatro casos a lesao localizava-se no corpo caloso e em um caso ocupava a regiao supra-selar. A queixa mais comum foi a cefaléia constante, presente em dois casos. Um caso apresentava buftalmo unilateral. O tratamento foi sintomático em todos os casos.